Niemann-Pick type C disease and intracellular cholesterol trafficking (Q34411567)

2 August 2017

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title

Niemann-Pick type C disease and intracellular cholesterol trafficking (English)

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2 August 2017

Ta-Yuan Chang

1

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2 August 2017

Patrick C Reid

2

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2 August 2017

Shigeki Sugii

3

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2 August 2017

Nobutaka Ohgami

4

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2 August 2017

Jonathan C Cruz

5

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2 August 2017

Catherine C Y Chang

6

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2 August 2017

language of work or name

English

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publication date

14 April 2005

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Journal of Biological Chemistry

2 August 2017

number of pages

4

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based on heuristic

inferred from page(s)

published in

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2 August 2017

volume

280

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2 August 2017

issue

22

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2 August 2017

page(s)

20917-20920

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Direct binding of cholesterol to the purified membrane region of SCAP: mechanism for a sterol-sensing domain

2 August 2017

cites work

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21 January 2018

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Niemann-Pick C1 Like 1 protein is critical for intestinal cholesterol absorption

21 January 2018

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Dynamic movements of organelles containing Niemann-Pick C1 protein: NPC1 involvement in late endocytic events

21 January 2018

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Binding between the Niemann-Pick C1 protein and a photoactivatable cholesterol analog requires a functional sterol-sensing domain

21 January 2018

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Transmembrane molecular pump activity of Niemann-Pick C1 protein

21 January 2018

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Determinants of NPC1 expression and action: key promoter regions, posttranscriptional control, and the importance of a "cysteine-rich" loop

21 January 2018

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Identification of 58 novel mutations in Niemann-Pick disease type C: correlation with biochemical phenotype and importance of PTC1-like domains in NPC1.

21 January 2018

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Sterol-modulated glycolipid sorting occurs in niemann-pick C1 late endosomes

21 January 2018

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Targeting of NPC1 to late endosomes involves multiple signals, including one residing within the putative sterol-sensing domain

21 January 2018

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Separation and characterization of late endosomal membrane domains

21 January 2018

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Identification of HE1 as the second gene of Niemann-Pick C disease

21 January 2018

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The integrity of a cholesterol-binding pocket in Niemann-Pick C2 protein is necessary to control lysosome cholesterol levels.

21 January 2018

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Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease

21 January 2018

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Distinct endosomal compartments in early trafficking of low density lipoprotein-derived cholesterol

21 January 2018

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The transport of low density lipoprotein-derived cholesterol to the plasma membrane is defective in NPC1 cells

21 January 2018

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NPC1 and NPC2 Regulate Cellular Cholesterol Homeostasis through Generation of Low Density Lipoprotein Cholesterol-derived Oxysterols

21 January 2018

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The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts

21 January 2018

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Characterization of rapid membrane internalization and recycling

21 January 2018

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Cholesterol movement in Niemann-Pick type C cells and in cells treated with amphiphiles

21 January 2018

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Trafficking defects in endogenously synthesized cholesterol in fibroblasts, macrophages, hepatocytes, and glial cells from Niemann-Pick type C1 mice

21 January 2018

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Thematic review series: brain Lipids. Cholesterol metabolism in the central nervous system during early development and in the mature animal

21 January 2018

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Trafficking of cholesterol from cell bodies to distal axons in Niemann Pick C1-deficient neurons

21 January 2018

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Cholesterol accumulates in cell bodies, but is decreased in distal axons, of Niemann-Pick C1-deficient neurons

21 January 2018

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Secretion of sterols and the NPC2 protein from primary astrocytes

21 January 2018

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Cholesterol modulates membrane traffic along the endocytic pathway in sphingolipid-storage diseases

21 January 2018

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Sphingolipid storage induces accumulation of intracellular cholesterol by stimulating SREBP-1 cleavage

21 January 2018

1 reference

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Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport

21 January 2018

1 reference

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Jamming the endosomal system: lipid rafts and lysosomal storage diseases.

21 January 2018

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Posttranslational regulation of acid sphingomyelinase in niemann-pick type C1 fibroblasts and free cholesterol-enriched chinese hamster ovary cells

21 January 2018

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Glucosylceramidase mass and subcellular localization are modulated by cholesterol in Niemann-Pick disease type C.

21 January 2018

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Mutagenesis of the putative sterol-sensing domain of yeast Niemann Pick C-related protein reveals a primordial role in subcellular sphingolipid distribution

21 January 2018

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Targeting Rab GTPases to distinct membrane compartments

21 January 2018

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Disturbed cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts

21 January 2018

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Late endosome motility depends on lipids via the small GTPase Rab7.

21 January 2018

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Elevated endosomal cholesterol levels in Niemann-Pick cells inhibit rab4 and perturb membrane recycling

21 January 2018

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Telomerase immortalization upregulates Rab9 expression and restores LDL cholesterol egress from Niemann-Pick C1 late endosomes

21 January 2018

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MENTHO, a MLN64 homologue devoid of the START domain

21 January 2018

1 reference

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Targeted mutation of the MLN64 START domain causes only modest alterations in cellular sterol metabolism

21 January 2018

1 reference

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Cholesterol overload promotes morphogenesis of a Niemann-Pick C (NPC)-like compartment independent of inhibition of NPC1 or HE1/NPC2 function

21 January 2018

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The ABCA1 transporter modulates late endocytic trafficking: insights from the correction of the genetic defect in Tangier disease

21 January 2018

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21 January 2018

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Cholesterol storage defect in RSH/Smith-Lemli-Opitz syndrome fibroblasts

21 January 2018

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Neurodegeneration in Niemann-Pick type C disease mice

21 January 2018

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A novel cholesterol stain reveals early neuronal cholesterol accumulation in the Niemann-Pick type C1 mouse brain

21 January 2018

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Perturbed myelination process of premyelinating oligodendrocyte in Niemann-Pick type C mouse

21 January 2018

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Rescue of neurodegeneration in Niemann-Pick C mice by a prion-promoter-driven Npc1 cDNA transgene

21 January 2018

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Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport

21 January 2018

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Critical role for glycosphingolipids in Niemann-Pick disease type C.

21 January 2018

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Neurosteroids: biosynthesis and function of these novel neuromodulators

21 January 2018

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Niemann-Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone

21 January 2018

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Fate of Endogenously Synthesized Cholesterol in Niemann-Pick Type C1 Cells

21 January 2018

1 reference

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Embryonic Striatal Neurons from Niemann-Pick Type C Mice Exhibit Defects in Cholesterol Metabolism and Neurotrophin Responsiveness

21 January 2018

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21 January 2018

Identifiers

DOI

10.1074/JBC.R400040200

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2 August 2017

1 reference